系统性硬化症下消化道受累13例

Low Gastrointestinal Involvement in Systemic Sclerosis:a Retrospective Study of 13 Chinese Inpatients

目的分析和总结系统性硬化症(systemic sclerosis,SSc)并发下消化道受累患者的临床特点。方法回顾性分析2000年11月至2015年6月北京协和医院住院SSc并发下消化道受累患者的临床、实验室、影像学和内镜检查结果、治疗和转归。结果 SSc住院患者并下消化道受累发生率约2.3%(13/569)。13例SSc并下消化道受累患者中男∶女=1∶1.25。弥漫性皮肤型SSc 6例,局限性皮肤型SSc 7例,重叠综合征2例。SSc并下消化道受累起病平均年龄(45±18)岁,从SSc起病到出现下消化道受累中位时间为5年,92%(12/13)下消化道症状出现在SSc其他系统首发表现之后,1例SSc患者以下消化道症状为首发表现。SSc并下消化道受累临床表现以肠梗阻最常见(8/13,62%),其次是肠溃疡、失蛋白肠病、小肠细菌过度生长、肛门括约肌受累。13例患者中6例失访,另7例随访中位时间20个月,病情平稳。结论 SSc并下消化道受累并非罕见,严重者将显著影响患者生活质量。对SSc患者及时行影像学和内镜检查,有利于早期发现下消化道病变。

Objective To analyze and summarize the clinical features of low gastrointestinal involvement in pa- tients with systemic sclerosis （SSc）. Methods Medical data of SSc patients with low gastrointestinal in- volvement of SSc admitted to Peking Union Medical College Hospital from November 2000 to June 2015 were retrospectively reviewed. The clinical manifestations, laboratory tests, image examinations and en- doscopic characteristics, treatments and outcomes of these patients were analyzed. Results The inci- dence of SSc inpatients with low gastrointestinal involvement was 2. 3%. Thirteen patients, including 9 women and 4 men were included. The median age of lower gastrointestinal involvement onset was （45 ± 18） years. It took 5 years from the diagnosis of SSc to low gastrointestinal involvement presentations in average. Six patients were with diffuse cutaneous systemic sclerosis, 7 were limited cutaneous systemic sclerosis, and 2 were overlap syndrome. In SSc, low gastrointestinal symptoms appeared as the secondclinical presentation in most patients （92%）, only one patient had low gastrointestinal symptom as the initial manifestation of SSc. Pseudo-obstruction （8/13, 62% ） was the most common manifestation, fol- lowed by intestinal ulcer, protein losing enteropathy, enteric bacterial overgrowth syndrome and anal sphincter dysfunction. Of the 13 patients, 6 patients were lost of follow-up. Among 7 patients with long- term followed-up, their overall condition was relatively stable. Conclusion Low gastrointestinal involve- ment in SSc is not uncommon, which may seriously affect the quality of life of patients. Preceding image examination and endoseopyare helpful to early diagnose of low gastrointestinal diseases.